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Immunology Flashcards

1
Q

a. What muscles come from first arch?

b. what nerves come from first arch?

A

a. muscles of mastication, mylohyoid muscle, tensor tympani, tensor veli palatini
b. maxillary and mandibular division of trigeminal nerve

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2
Q

What amino acids are found in elastin?

A

Glycine and proline

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3
Q

What is mechanism of adenosine as anti-arrhythmic?

A

Increases outward K current
Hyper polarization of cells
Decreases intracellular Ca

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4
Q

What part of the body drains to:

a. Axillary lymph node
b. Superificial inguinal nodes
c. Popliteal nodes

A

a. Upper limb, breast, skin above umbilicus
b. anal canal (below pectinate line), skin below umbilicus, scrotum
c. Dorsolateral foot, posterior calf

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5
Q

Which part of the GI tract drains to:

a. Celiac lymph nodes
b. Superior mesenteric nodes
c. Inferior mesenteric nodes
d. Internal iliac nodes

A

a. liver, stomach, spleen, pancreas, upper duodenum
b. lower duodenum, jejunum, ileum, colon to splenic flexure
c. colon from splenic flexure to upper rectum
d. lower rectum to anal canal (above pectinate line), bladder, vagina, prostate

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6
Q

Which lymph nodes do the testes drain to? the scrotum?

A

Testes –> PARA-AORTIC

Scrotum –> superficial inguinal

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7
Q

What part of the body does the right lymphatic duct drain?

A

Right side of body above the diaphragm

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8
Q

What part of the body does the thoracic duct drain?

A

Everything else besides right side of body above diaphragm. Goes into junction of left subclavian and internal jugular veins.

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9
Q

Where do you find peer’s patches?

A

In lamina propria submucosa of ileum

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10
Q

a. What muscles are derived from second branchial arch?

b. nerves from second branchial arch?

A

a. Muscles of facial expression, stapedius, stylohyoid

b. Cranial nerve seven

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11
Q

Which cells express MHCI?

A

ALL cells EXCEPT RBCs

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12
Q

a. What genes code for MHC I?

b. What genes code for MHC II?

A

a. HLA-A, B and C

b. HLA-DR, DP, and DQ

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13
Q

What diseases does HLA-B27 predispose to?

A 
PAIR
Psoriatic arthritis
Ankylosing spondylitis
Inflammatory Bowel Disease
Reactive arthritis
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14
Q

What diseases do HLADR3/4 predispose to?

A

Diabetes mellitus type I

DR4 - Rheumatoid arthritis

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15
Q

a. What is Langerhans Cell Histiocytosis?
b. What markers does it express?
c. ON electron microscopy?
d. How does it present?

A

a. Group of proliferative disorders of dendritic cells
b. Express S-100 and CD1a
c. Birbeck granules
d. Presents as child with lytic bone lesion and skin rash or recurrent otitis media with mass involving mastoid bone

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16
Q

What 4 things are expressed on dendritic cells?

A

B7
CD40
MHC I
MHC II

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17
Q

What mediates the following reactions:

a. hyperacute transplant rejection
b. acute transplant rejection
c. chronic rejection
d. graft vs. host

A

a. pre-existing recipient antibodies
b. T cells
c. T cells and Antibodies; Cytotoxic T cells treat transplant cells as self cells presenting non-self antigen - irreversible
d. Usually in BM transplants; the grafted immunocompetent T cells proliferate in the immunocompromised host and reject host cells with “foreign proteins”

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18
Q

How does graft vs. host disease present?

A

Maculopapular rash, jaundice

Diarrhea, hepatosplenomegaly

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19
Q

Cyclosporine

a. Mechanism
b. Uses
c. Adverse effects (unique one?)

A

a. Binds to cyclophilin –> inhibits Calcineurin –> prevents production of IL2 –> disrupts T cell activation
b. Prevent transplant rejection, psoriasis, RA
c. Viral infections, lymphomas, NEPHROTOXICITY, HTN

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20
Q

Tacrolimus

a. Mechanism
b. Uses
c. Adverse effects

A

a. Bind FK506 binding protein (FKBP) –> inhibits Calcineurin –> prevents production of IL2 –> blocks T cell activation
b. Transplant rejection prophylaxis
c. Increased risk of diabetes and neurotoxicity,, nephrotoxicity, HTN

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21
Q

Sirolimus

a. Mechanism
b. Uses
c. Adverse effects

A

a. binds FKBP –> inhibits mTOR –> inhibition of T cell proliferation and prevents response to IL2
b. Kidney transplant
c. Not that important - NOT NEPHROTOXIC; anemia, thrombocytopenia, leukopenia

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22
Q

Azathioprine

a. Mechanism
b. Use
c. Adverse effects

A

(precursor to 6-Mercaptopurine)

a. Inhibits lymphocyte proliferation by blocking nucleotide synthesis
b. Transplant rejection prophylaxis (especially kidney), RA, glomerulonephritis
c. BONE MARROW suppression; degraded by Xanthine Oxidase (toxic effects can be increased by Allopurinol)

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23
Q

Mycophenolate

a. Mechanism
b. Use

A

a. Inhibits IMP dehydrogenase –> prevents synthesis of guanine –> no cell replication/proliferation of B cells and T cells
b. Transplant patients and Lupus nephritis

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24
Q

Muromonab

a. Mechanism
b. Use

A

a. Binds CD3 (T cells) –> interferes with T cell signal transduction –> shuts down T cells
b. Transplants

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25
Daclizumab a. Mechanism b. Use
a. Binds to CD25 (IL2 receptor on activated T cells) --> blocks IL-2 signaling (Sirolimus is similar)
26
Thalidomide a. mechanism b. use c. toxicity
a. affects TNFa b. Immunosuppression, anti-angiogenic c. NOT USED - phocomelia, teratogen
27
a. What are the anti-TNFa agents? b. What are they used for? c. Which anti-TNF alpha drug is not a monoclonal antibody?
a. Adalimumab, Infliximab b. Ankylosing spondylitis, RA, IBD, reactive arthritis c. Etanercept - mimics TNFa receptor (gobbles up TNFa in circulation)
28
Abciximab a. Mechanism b. Use
a. Targets platelet glycoproteins IIb/IIIa b. Anti-platelet agent for prevention of ischemic complications in patients undergoing percutaneous coronary intervention
29
Trastuzumab (Herceptin) a. Mechanism b. Use
a. Ab against HER2 | b. Breast cancer that expresses HER2
30
Rituximab a. Mech b. Use
a. Targets CD20 | b. B cell NON-Hodgkin lymphoma
31
Omalizumab a. Mech b. Use
a. Targets IgE | b. Severe asthma
32
Bruton agammaglobulinemia a. defect b. presentation c. findings
a. defect in BTK (tyrosine kinase gene) --> no B cell maturation; X linked recessive b. Recurrent bacterial infections and enteroviral infections after 6 months (decreased maternal IgG) c. Absent B cells in peripheral blood, decrease IgG of ALL classes; absent lymph nodes/tonsils
33
Selective Ig Deficiency a. Defect b. Presentation c. Findings
a. Most common primary immunodeficiency; unknown b. Majority asymptomatic, can see airway and GI infections; autoimmune disease (atopy, asthma) anaphylaxis to IgA products c. Decreased IgA with normal IgG and IgM
34
Common Variable Immunodeficiency a. Defect b. Presentation c. Findings
a. Defect in B cell differentiation b. Can be acquired in 20-30s, increased risk of autoimmune disease, bronchiectasis, lymphoma, sinopulmonary infections c. decreased plasma cells, decreased immunoglobulins
35
Thymic aplasia (DiGeorge) a. defect b. presentation c. findings
a. 22q11 deletion; failure to develop 3rd and 4th pharyngeal pouches --> absent parathyroids and thymus b. Cardiac abnormality, Abnormal facies, Thymic aplasia, Cleft palate, Hypocalcemia - recurrent viral, fungal and protozoal infections c. Decreased T cells, PTH and Calcium; absent thyme shadow on CXR
36
IL-12 receptor deficiency a. defect b. presentation c. findings
a. decreased TH1 response; AR b. disseminated MYCOBACTERIAL and fungal infections; c. Decreased IFNy
37
Chronic Mucocutaneous Candidiases a. defect b. presentation c. findings
a. T cell dysfunction; many causes b. non-invasive Candida albicans infections of skin and mucous membranes c. absent cutaneous reaction and T cell proliferation in response to Candida antigens
38
Hyper IgM Syndrome a. defect b. presentation c. findings
a. defective CD40L on Th cells --> defect in class switching; X linked recessive (3 types) b. Severe pyogenic infections early in life; opportunistic infection with Penumocystis, Crypto, CMV c. Increased IgM, decreased other antibodies
39
SCID a. defect b. presentation c. findings
a. defect in early stem cell differentiation; caused by many including adenosine deaminase deficiency (X linked) b. failure to thrive, chronic diarrhea, thrush, recurrent viral/bacterial/fungal/protozoal infections (treat with BM transplant) c. Decreased T cell receptor excision circles (TRECs); absence of thymic shadow, germinal centers, and T cells
40
Wiskott-Aldrich Syndrome a. defect b. presentatin c. findings
a. Mutation in WAS gene (X linked) --> T cells unable to recognize actin cytoskeleton b. Thrombocytopenia purpura, Eczema (on trunk), Recurrent infections c. Decreased to normal IgG, IgM; Increased IgE and IgA; fewer/smaller platelets
41
What are the X linked immunodeficiencies?
Wiskott Aldrich Bruton Agammaglobulinemia Chronic granulomatous disease Hyper-IgM syndrome
42
Ataxia Telangiectasia a. defect b. presentation c. findings
a. defects in ATM gene --> failure to repair DNA double strand breaks --> cell cycle arrest b. Triad = Cerebellar defects (ATAXIA), spider Angiomas (telangiectasia) IgA deficiency c. Increased AFP, decreased IgA, IgG an IgE; lymphopenia, cerebellar atrophy
43
Chronic Granulomatous Disease a. defect b. presentation c. findings
a. defect in NADPH oxidase --> decreased reactive oxygen species and respiratory burst in neutrophils b. Increased susceptibility to catalase + organisms (Nocardia, Pseudomonas, Listeria, Aspergillus, Candida, E. coli, S. aureus, Serratia c. Abnormal dihydroorhodamine test; nitroblue tetrazolium dye reduction test is negative
44
How is CGD treated?
Prophylactic TMP-SMX
45
Chediak-Higashi Disease a. defect b. presentation c. findings
a. defective LYST gene --> defective lysosomal transport --> microtubule dysfunction in pharosome-lysosome fusion (AR) b. Recurrent pyogenic infections by Staph and Strep; partial albinism, peripheral neuropathy, progressive neurodegeneration, infiltrative lymphohistiocytosis c. giant granules in granulocytes and platelets, pancytopenia, coagulation defects
46
Leukocyte adhesion deficiency type 1 a. defect b. presentation c. findings
a. defect in LFA-1 integrin (CD18) on phagocytes, impaired migration and chemotaxis (AR) b. recurrent bacterial skin and mucosal infections, absent pus formation, impaired wound healing, delayed separation of umbilical cord c. Increased neutrophils, absence of neutrophils at infection sites
47
What cytokines do T helper cells produce to stimulate B cells?
IL4, IL5
48
What are the surface markers of B cells?
CD19, CD20, CD21, IgM, IgD
49
What cell markers do T cells have?
CD3, CD28, T cell receptor Cytotoxic T cells have CD8 Helper T cells have CD4 and CD40L
50
What cell markers do NK cells have?
CD56 and CD16
51
What cell markers do macrophages have?
CD14, MHC II, B7 protein, CD40
52
Which organism's exotoxin works via each of the following mechanisms? a. inactivates EF2 b. activates Gs c. Disables Gi d. Bacterial adenylat cyclase (No G protein involved) e. Blocks GABA and glycine
a. Corynebacterium diptheriae, Pseudomonas (exotoxin A) b. Vibrio cholerae, E. coli (ETEC heat labile toxin) c. Pertussis toxin d. Bacillus anthracis e. Clostridium tetani (lockjaw)
53
What drug helps to make diagnosis of Myasthenia gravis?
Edrophonium (acetylcholinesterase inhibitor) | Too short acting to treat
54
What heart sound is associated with dilated CHF?
S3
55
What heart sound is associated with stiffened LV?
S4
56
What is amyloidosis?
Accumulation of one specific protein in the tissues that aggregates into B pleated sheets which causes damage and apoptosis
57
What is AL amyloidosis? What protein is deposited? Which organs does it affect?
(Primary) Due to deposition of proteins from Ig Light chains Can occurs as plasma cell disorder or associated with multiple myeloma. Often affects multiple organ systems including renal (nephrotic syndrome), cardiac (restrictive CM, arrhythmia), hematologic (brushing, splenomegaly), GI (hepatomegaly) and neurologic (neuropathy)
58
What is AA amyloidosis? What protein is deposited? What is it associated with?
(Secondary) Seen in chronic inflammatory conditions such as RA, IBD, spondyloarthropathy Fibrils composed of serum Amyloid A; often multi system
59
a. Patient with fibrils made of B2 microglobulin, ESRD and/or long term dialysis. b. What else may it present as?
a. Dialysis-related amyloidosis | b. Carpal tunnel syndrome
60
Which protein and fibril with each form of amyloidosis? a. type 2 diabetes b. Medullary thyroid CA c. Alzheimer d. Dialysis -associated
a. amylin, AIAPP b. Calcitonin, A-CAL c. B-amyloid, APP d. B2 microglobulin, AB2M
61
How do you diagnosis amyloidosis on biopsy?
Congo red stain; apple green birefringence
62
Disease associated with anti-IgG antibodies
Rheumatoid arthritis
63
Disease associated with anti-citrullinated protein antibodies (ACPA)
RA
64
Associated with anti-centromere ABs
CREST Scleroderma | C for centromere, C for CREST
65
Associated with Anti-Scl-70 Abs
``` Diffuse Scleroderma (Scl = Scleroderma) ```
66
Associated with Anti-histone Abs
Drug-induced Lupus
67
Associated with anti-nuclear Abs (ANA)
SLE
68
Associated with Anti-smith Abs
SLE
69
Associated with Anti-dsDNA Abs
Lupus nephritis
70
Associated with Anti-Jo-1 Abs
Polymyositis/Dermatomyositis
71
Associated with Anti-SSA (anti-Ro)
Sjogren syndrome
72
Associated with Anti-SSB (anti-La)
Sjogren syndrome
73
Associated with Anti-U1-RNP Abs
Mixed CT disease
74
Associated with Anti-desmoglein Abs
Pemphigus vulgaris
75
Associated with Anti-acetylcholine receptor Abs
Myasthenia gravis
76
Associated with Anti-endomysial (anti-tissue transglutaminase)
Celiac disease
77
Associated with Anti-gliadin Abs
Celiac disease
78
Associated with anti-mitochondrial Abs
Primary Biliary Cirrhosis
79
Associated with Anti-smooth muscle Abs
Autoimmune Hepatitis
80
Associated with anti-glutamate decarboxylase Abs
Type 1 Diabetes
81
Associated with Anti-thyrotropin receptor Abs
Graves disease
82
Associated with Anti-thyroid peroxidase (anti-TPO)
Hashimoto thyroiditis
83
Anti-thyroglobulin
Hashimoto thyroiditis, Graves disease
84
Anti-Basement membrane Abs
Goodpasture syndrome
85
c-ANCA
Granulomatosis with polyangiitis (Wegener's)
86
p-ANCA
Pauci-immune crescentic glomerulonephritis Churg-Strauss syndrome Microscopic polyangiitis
87
Which vaccines are dangerous for patients with egg allergy?
Influenza | Yellow fever
88
What mediates Type I Hypersensitivity?
Free antigen cross-linkes IgE on presensitized mast cells and basophils --> triggers immediate release of vasoactive amines that act on post capillary venules (histamine and bradykinin)
89
What mediates Type II Hypersensitivity?
Cytotoxic (Ab mediated); IgM an IgG bind to FIXED antigen on "enemy" cell and cause cellular destruction by opsonization/phagocytosis, complement mediated inflammation or Ab-mediated cellular dysfunction
90
What is Serum sickness?
Type III hypersensitivity reaction where antibodies to foreign proteins are produced --> immune complexes form and are deposited in membranes where they fix complement (cause tissue damage); mostly caused by drugs; happens 5-10 days after antigen exposure
91
What are signs of serum sickness?
Fever, urticaria, arthralgia, proteinuria, lymphadenopathy
92
What is Arthus reaction?
Local subacute antibody mediated type III hypersensitivity reaction caused by intradermal injection of antigen; takes 4-12 hours
93
What are signs of Arthus reaction?
Edema, necrosis, activation of complement
94
Acute hemolytic transfusion reaction
Type II
95
Acute rheumatic fever
Type II
96
Arthus reaction
Type III
97
Asthma
Type I
98
Bee sting allergy
Type I
99
Contact dermatitis
Type IV
100
Eczema
Type I
101
Graves disease
Type II
102
Guillain Barre syndrome
Type IV
103
GVHD
Type IV
104
Hashimoto thyroiditis
Type IV
105
ITP
Type II
106
MS
Type IV
107
Myasthenia gravis
Type II
108
PAN
Type III
109
Peanut allergy
Type I
110
Poststreptococcal GN
Type III
111
RA
Type III
112
Seasonal allergic rhinitis
Type I
113
SLE
Type III
114
What is the starting point for the Classic complement pathway?
IgG or IgM recognize C1
115
What is C3b important for?
Opsonization
116
What are the two most important opsonins?
C3b and IgG
117
What are C3a and C5a important for?
Anaphylaxis C3a - stimulates mast cells and basophils C5a - neutrophil chemotaxis
118
a. What happens if you are deficient in C1 esterase inhibitor? b. What drug is C/I?
a. Hereditary angioedema (from elevated Bradykinin) | b. ACE inhibitors are C/I
119
What happens if you are deficient in C3?
Recurrent pyogenic sinus and respiratory infections (Strep pneumo and H influenzae) Also increased susceptibility to type III hypersensitivity and pyelonephritis
120
What happens if you are deficient in C5b-C9 proteins?
Susceptible to recurrent Neisseria bacteremia
121
a. What is DAF? | b. What happens if you have DAF deficiency?
a. GPI anchored enzyme (CD55) | b. Causes complement mediated lysis of RBCs and Paroxysmal Nocturnal Hemoglobinuria
122
What kinds of organisms are asplenic patients susceptible to?
Encapsulated bacteria
123
Even Some Pretty Nasty Killers Have Shiny Bodies
``` E. coli Streptococcus pneumo Pseudomonas aeruginosa Neisseria meningitidis Klebsiella pneumoniae Haemophilus influenza Salmonella typhi Group B Streptococcus ```
124
Why would patient be missing spleen?
Trauma Sickle cell patient Hereditary spherocytosis - RBCs are defective and spleen keeps removing them
125
What are macrophages activated by? What cells produce it?
IFNy | Produced by TH1 and NK cells
126
Which cytokines do macrophages make?
IL1 IL6 TNFa
127
What does IL-12 do?
Induces differentiation of T cells into TH1 cells | Activates NK cells
128
What are the neutrophil chemotactic factors?
IL8, C5a, leukotriene B4
129
What is Aldesleukin? What is it used for?
Recombinant IL-2 used for renal cell carcinoma and metastatic melanoma
130
What are Filgrastim and Sargramostim? What are they used for?
Recombinant GM-CSF used for recovery of bone marrow
131
What is Oprelvekin?
Recombinant IL-11 used for thromobocytopenia
132
What is recombinant IFN-a used for?
Hepatitis B/C, Kaposi sarcoma, malignant melanoma, genital warts, hairy cell leukemia
133
What is recombinant IFN-b used for?
Multiple sclerosis
134
What is recombinant IFN-y used for
Chronic Granulomatous Disease
135
What cytokines do TH1 cells produce?
IL-2 and IFNy
136
What cytokine inhibits macrophage activation?
IL-10
137
What does IL-10 inhibit?
Production of TH1 cells and macrophage activation
138
What inhibits production of TH2 cells?
IFNy
139
What muscles and nerves are derived from third branchial arch?
``` Stylopharyngeus muscle Glossopharyngeal nerve (IX) ```
140
What kind of drug is used in treatment of Myasthenia gravis?
Acetylcholinesterase inhibitors like Pyridostigmine, Physostigmine
141
What cytokines enhance NK cell activity?
IL-12 IL-2 IFN-a IFN-b
142
What do patients with common variable immunodeficiency have increased risk of later in life?
Autoimmune disease and lymphoma
143
Complications of Sjogren's syndrome
``` Dental caries (decreased saliva production) mucosa associated lymphoid tissue lymphoma (UNILATERAL PAROTID ENLARGEMENT) ```

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